Asad, Murva

Mallon, Dermot

Mehta, Arpan R

English

UCL Discovery

1  Fulminant MRI brain abnormali0es in a rare neurological presenta0on of Kikuchi-Fujimoto disease.  Murva Asad MB BCh BAO 1,4, Arpan R. Mehta PhD 1,2,3, Dermot Mallon PhD 1,4 1. Na<onal Hospital for Neurology and Neurosurgery, Queen Square, London, UK 2. Oxford University Hospitals NHS Founda<on Trust, Oxford, UK 3. Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, UK 4. UCL Queen Square Ins<tute of Neurology, Queen Square, London, UK  Corresponding author: Dr Murva Asad, Centre for Neuromuscular Diseases National Hospital for Neurology and Neurosurgery Queen Square London WC1N 3BG e-mail: murva.asad@ucl.ac.uk Word count: 507 Date of revision: 21st Jan 2024  2   A previously well 17-year-old male presented with reduced consciousness and dystonic storm requiring emergency intuba<on and ven<la<on. He had had a six-week prodrome of high fevers, anorexia, and cervical lymphadenopathy, ini<ally diagnosed as infec<ous mononucleosis.  Examina<on revealed fluctua<ng alertness, bruxism, intermi[ent opisthotonos, extensor posturing of his limbs and upgoing plantar responses. He had an enlarged paro<d gland and tender cervical lymphadenopathy.  Treatment for suspected central nervous system infec<on with broad-spectrum an<microbials was ini<ated empirically without clinical improvement.  Blood tests revealed mild anaemia, hyperferri<naemia, transamini<s, raised erythrocyte sedimenta<on rate and a posi<ve speckled an<nuclear an<body (<tre 1:160 with nega<ve dsDNA and ENA an<bodies). Epstein Barr virus (EBV) IgM was ini<ally weakly posi<ve, however EBV PCR was nega<ve and viral load was not detected. An<body to EBV nuclear an<gen was ini<ally weakly reac<ve and subsequently nega<ve. Ini<al an<-viral capsid an<gen (VCA) IgM was equivocal and an<-VCA IgG was nega<ve. Evidence for acute EBV infec<on was therefore thought not to be substan<ve. Other serum metabolic, infec<ous, vasculi<s and autoimmune screens were nega<ve. CSF analysis, including viral PCR and bacterial/fungal cultures, was unremarkable.  MRI brain revealed extensive T2-weighted hyperintensity, focal diffusion restric<on, and microhaemorrhages within the deep grey nuclei and surrounding white ma[er (Fig 1). An 3  FDG-PET/CT scan showed increased uptake within enlarged cervical lymph nodes and the spleen. A wide differen<al was considered, including lymphoma, hemophagocy<c lymphohis<ocytosis (with no response to anakinra and ul<mately no haemophagocystosis on biopsy), bio<n-thiamine-responsive basal ganglia disease (with no response to bio<n and thiamine replacement), post-infec<ous acute necro<sing encephalopathy and gene<c acute necro<sing encephalopathy (RANBP2 gene analysis was normal).   Tissue samples were obtained given the diagnos<c uncertainty. Bone marrow trephine and skin biopsy did not suggest a haematological malignancy. Cervical lymph node biopsy (Fig. 2) showed vast areas of necrosis with karyorrhec<c debris, his<ocytes with crescen<c nuclei and very few neutrophils, with a predominance of CD8+ T-cells. This confirmed the diagnosis of his<ocy<c necro<zing lymphadeni<s or Kikuchi-Fujimoto disease (KFD). The pa<ent was treated with high dose intravenous methylprednisolone followed by a slow steroid taper. This led to significant improvement; shortly aier introduc<on of steroids, he began to vocalise and obey commands. On discharge, aier five months of neurorehabilita<on, he was independently mobile with improving bulbar func<on.  KFD is a rare idiopathic disease that is usually self-limi<ng. Viral triggers and an associa<on with systemic lupus erythematosus have been suggested [1]. Encephalopathy in KFD is uncommon - one study found nine cases reported in children between 2010 and 2020 [2]. Another large case series reported an incidence of neurological complica<ons of 4.5% in 4  KFD, most commonly asep<c meningi<s. Headaches, meningism, seizures, confusion and ataxia were reported 10-53 days aier onset of classical fever and cervical lymphadenopathy [3]. Reports of brain lesions on MRI are sparse; two other cases with bilateral basal ganglia changes have been documented in children. Others report leptomeningeal, brainstem, cerebellar, occipital or temporal lobe changes. CSF analysis may be normal or may show pleocytosis and elevated protein [4]. Lack of familiarity with this rare condi<on can result in diagnos<c delays and inappropriate treatment.   5  Figure legends: Figure 1. A. Axial T2-weighted imaging of the brain showing extensive hyperintensity and swelling of the basal ganglia and surrounding white ma[er. B. Suscep<bility-weighted imaging showing microhaemorrhages.  Figure 2. Photomicrograph of haematoxylin and eosin-stained lymph node biopsy sample, demonstra<ng large areas of necrosis, karyorrhec<c debris (blue arrows) and his<ocytes with crescen<c nuclei (yellow arrows).             6  References: 1. Santana A, Lessa B, Galrão L, Lima I, Santiago M. Kikuchi-Fujimoto’s disease associated with systemic lupus erythematosus: Case report and review of the literature. Clinical Rheumatology. 2004;24(1):60-63. doi:10.1007/s10067-004-0923-6  2. Pan Y-T, Cao L-M, Xu Y, Fan Z-D, Yu H-G. Kikuchi-Fujimoto Disease with encephalopathy in children : Case reports and literature review. Frontiers in Pediatrics. 2021;9. doi:10.3389/fped.2021.727411  3. Kucukardali Y, Solmazgul E, Kunter E, Oncul O, Yildirim S, Kaplan M. Kikuchi–Fujimoto Disease: Analysis of 244 cases. Clinical Rheumatology. 2006;26(1):50-54. doi:10.1007/s10067-006-0230-5  4. Huang Y, Cheng M, Tang C, Wang J, Wang L, Wang X. Kikuchi–Fujimoto disease associated with the central nervous system in children. Pediatric discovery. 2023;1(2). doi:10.1002/pdi3.23         7   Figure 1  8   Figure 2 

A Rare Neurological Presentation of Kikuchi-Fujimoto Disease

https://discovery.ucl.ac.uk/10189299/2/Asad_A%20rare%20neurological%20presentation%20of%20Kikuchi-Fujimoto%20disease_AAM.pdf

A Rare Neurological Presentation of Kikuchi-Fujimoto Disease

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